[Cystic fibrosis in adults]. 1995

I Durieu, and G Bellon, and D Vital Durand, and L Calemard, and Y Morel, and R Gilly
Service de Médecine interne, Centre Hospitalier Lyon-Sud, Pierre-Bénite.

OBJECTIVE At least half cystic fibrosis patients now reach adulthood. METHODS We report a population of 61 patients above 18 years of age with the clinical pictures at time of diagnosis and the present clinical status. RESULTS Thirty-five males and 26 females are aged from 18 to 47 years. Mean age at time of diagnosis was 5 years and 5 months, under 10 years in 80% of patients and above 15 years in 9 patients. Diagnosis was suspected because of pulmonary (2/3) or digestive (1/3) symptoms, insufficient height and weight (1/3) or past family history of cystic fibrosis (1/3). 37% of patients are homozygotes for delta F508 mutation. Adult patients had a normal height but half of them a body weight under 90% of expected weight. Recurrent pulmonary infections were observed in 95% of patients and 62% have chronically infected sputum with Pseudomonas aeruginosa. These patients had lower weight and a poorer radiological score than patients without pseudomonas. 25% of all patients had chronic respiratory insufficiency. 75% had pancreatic insufficiency and 6 patients diabetes mellitus. Thirteen patients had biological cholestasis and three a liver cirrhosis with portal hypertension. Four women underwent 6 normal pregnancies; semen analysis in five men revealed aspermia. Seven patients died during the last two years because of respiratory insufficiency (4), in the three months after pulmonary transplantation (2), and because of digestive haemorrhage (1). CONCLUSIONS Treatment included daily bronchial drainage, adapted antibiotic treatment and pancreatic enzyme substitution.

UI MeSH Term Description Entries
D008171 Lung Diseases Pathological processes involving any part of the LUNG. Pulmonary Diseases,Disease, Pulmonary,Diseases, Pulmonary,Pulmonary Disease,Disease, Lung,Diseases, Lung,Lung Disease
D008297 Male Males
D008875 Middle Aged An adult aged 45 - 64 years. Middle Age
D010188 Exocrine Pancreatic Insufficiency A malabsorption condition resulting from greater than 10% reduction in the secretion of pancreatic digestive enzymes (LIPASE; PROTEASES; and AMYLASE) by the EXOCRINE PANCREAS into the DUODENUM. This condition is often associated with CYSTIC FIBROSIS and with chronic PANCREATITIS. Pancreatic Insufficiency,Exocrine Pancreatic Insufficiencies,Insufficiencies, Exocrine Pancreatic,Insufficiencies, Pancreatic,Insufficiency, Exocrine Pancreatic,Insufficiency, Pancreatic,Pancreatic Insufficiencies,Pancreatic Insufficiencies, Exocrine,Pancreatic Insufficiency, Exocrine
D011247 Pregnancy The status during which female mammals carry their developing young (EMBRYOS or FETUSES) in utero before birth, beginning from FERTILIZATION to BIRTH. Gestation,Pregnancies
D011550 Pseudomonas aeruginosa A species of gram-negative, aerobic, rod-shaped bacteria commonly isolated from clinical specimens (wound, burn, and urinary tract infections). It is also found widely distributed in soil and water. P. aeruginosa is a major agent of nosocomial infection. Bacillus aeruginosus,Bacillus pyocyaneus,Bacterium aeruginosum,Bacterium pyocyaneum,Micrococcus pyocyaneus,Pseudomonas polycolor,Pseudomonas pyocyanea
D011552 Pseudomonas Infections Infections with bacteria of the genus PSEUDOMONAS. Infections, Pseudomonas,Pseudomonas aeruginosa Infection,Infection, Pseudomonas,Pseudomonas Infection,Pseudomonas aeruginosa Infections
D011665 Pulmonary Valve Insufficiency Backflow of blood from the PULMONARY ARTERY into the RIGHT VENTRICLE due to imperfect closure of the PULMONARY VALVE. Pulmonary Regurgitation,Pulmonary Valve Incompetence,Incompetence, Pulmonary Valve,Insufficiency, Pulmonary Valve,Pulmonary Valve Regurgitation,Regurgitation, Pulmonary,Regurgitation, Pulmonary Valve,Valve Incompetence, Pulmonary,Valve Insufficiency, Pulmonary,Valve Regurgitation, Pulmonary
D001835 Body Weight The mass or quantity of heaviness of an individual. It is expressed by units of pounds or kilograms. Body Weights,Weight, Body,Weights, Body
D002779 Cholestasis Impairment of bile flow due to obstruction in small bile ducts (INTRAHEPATIC CHOLESTASIS) or obstruction in large bile ducts (EXTRAHEPATIC CHOLESTASIS). Bile Duct Obstruction,Biliary Stasis,Bile Duct Obstructions,Biliary Stases,Cholestases,Duct Obstruction, Bile,Duct Obstructions, Bile,Obstruction, Bile Duct,Obstructions, Bile Duct,Stases, Biliary,Stasis, Biliary

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